Dr. Sanjida Khan-1 for radiology residents.pptx

Congenital heart disease

1. Congenital Heart Diseases DR . SANJIDA KHAN MD Resident, Phase – B, Department of Radiology & Imaging, Sylhet MAG Osmani Medical College.

2. Development of heart Cardiac looping, cephalic portion bends caudally ,ventrally and towards right

3. Development of septum

4. Fetal circulation

5. Approach to congenital heart disease

6. Cardiac situs Situs solitus Situs inversus Situs ambiguous

7. Classification Cyanotic congenital heart disease Acyanotic congenital heart disease Increase pulmonary vascularity Total anomalous pulmonary venous return( TAPVR type I and II) Transposition of great arteries(TGA) Truncus arteriosus(Type I,II and III) Large AVSD Decreased pulmonary vascularity Tetralogy of Fallot Ebstein anomaly with ASD Double outlet right ventricle with pulmonary stenosis. Single ventricle with pulmonary stenosis Increased pulmonary vascularity Ventricular septal defect Atrial septal defect Patent ductus arteriosus Atrioventricular septal defect Partial anomalous pulmonary venous return(PAPVR) Normal vascularity Aortic stenosis Aortic coarctation Pulmonary stenosis

8. Imaging modalities NON-INVASIVE Plain radiograph Echocardiography with Doppler study Computed Tomography Magnetic Resonance Imaging Radio nuclide imaging INVASIVE Cardiac catheterisation and angiography

9. Ventricular septal defect (VSD) C ommonest of all CHDs, incidence is 36.1% Cardiovascular associations Tetralogy of fallot (TOF) Truncus arteriosus Double outlet right ventricle, Coarctation of aorta,Tricuspid atresia,Aortic regurgitation,Pulmonary stenosis

10. Classification Perimembranous defect Commonest type, Involving the membranous septum and adjacent muscular tissue below the aortic root and close to the upper margin of the tricuspid valve annulus. Types: a)anterior b)posterior c) supracristal and infracristal Muscular defects Inlet or basal muscular defect lying in the muscular septum between the mitral and tricuspid valves. Mid-muscular or apical defect between the main right and left ventricular chambers . Outlet or conal defect

11. CLINICAL PRESENTATION SYMPTOMS : The presentation of this condition depends on the overall size of interventricular communication- SMALL SIZED VSD(0.5cm) : asymptomatic usually,but may present at much later in life. LARGE VSD(>1cm) : asymptomatic at birth,may become symptomatic after 2-3 weeks of life with breathlessness,feeding difficulties,poor growth,palpitation,recurrent chest infection etc.

12. HAEMODYNAMICS Left ventricle ↓ VSD ( left to right shunt ) ↓ RV overload→ RV enlargement LV enlargement due to overactivity ↓ Increased Pulmonary artery flow→ PA enlargement. Eisenmenger,s syndrome ↓ ↑ Increased Pulmonary capillary flow→ Pulmonary plethora RV- LV sunt ↓ ↑ Pulmonary capillary damage Pressure of RV>LV ↓ ↑ Increased Pulmonary artery pressure ---------------------------→ PA hypertension

13. Xray findings Small – Normal h eart size and vascularity Moderate or large- 1.Cardiomegaly 2.Chamber enlargement- RV,LV (apex is rounded & sift to left) LA in late stage 3. Pulmonary bay full or bulged 4. Plethoric lung field-Enlarged pulmonary vessels Upper lobar diversion Vessels visible to the periphery of the film 5. Enlarged right and left pulmonary arteries 6.Eisenmenger reaction- large central arteries with disproportionately small peripheral arteries (pruning), heart size may be normal.

14. Ventricular septal defect (VSD) Two-dimensional echocardiography Doppler flow assessment detect the presence of of small defects,using the turbulent jet passing through the defect as a marker. Computed Tomography CT Angiogram with ECG-gating allows direct visualization of the defect. MRI May also show added functional information (e.g. quantification/shunt severity) in addition to anatomy.

15. Ventricular septal defect (VSD) Cardiac catheterization and angiography if there is any doubt about the intracardiac anatomy or about the nature of the pulmonary vascular resistance.

16. Ventricular septal defect (VSD Complications Eisenmenger phenomenon Congestive heart failure Secondary aortic insufficiency Aortic regurgitation Infective endocarditis Recurrent respiratory tract infections Treatment Small defects may be left for some years(as long as no significant pulmonary hypertension) to see if spontaneous closure occurs. Larger defects : surgical / medical treatment

17. Atrial septal defect (ASD) Second most common CHD after VSD, Incidence is 8.2% Due to the low pressures of the atria, the lesion is typically asymptomatic until adulthood. Gradual (high output) congestive cardiac failure eventually develops, usually becoming symptomatic by the age of 30. More common in female .

18. Types Ostium primum ASD Ostium secundum ASD (more common) Sinus venosus defect Associations ASDs usually tend to be isolated anomalies, of which its associations include: Down syndrome  (ostium primum defect) Holt- Oram syndrome Mitral valve prolapse Anomalous pulmonary venous return -TAPVR, PAPVR

19. Clinical presentation Typically asymptomatic until adulthood. As cardiac failure develops they may present with shortness of breath,dyspnea palpitations and weakness,repeated respiratory infection . Chest auscultation classically reveals an ejection systolic murmur

20. HAEMODYNAMICS Left atrium ↓ASD RA( left to right shunt ) Increased RA volume → RA enlargement RV overload→ RV enlargement ↓ Increased Pulmonary artery flow→ PA enlargement. Eisenmenger,s syndrome ↓ ↑ Increased Pulmonary capillary flow→ Pulmonary plethora RA- LA sunt ↓ ↑ Pulmonary capillary damage Pressure of RA>LA ↓ ↑ Increased Pulmonary artery pressure ----------------------------→ PA hypertension

21. Plain radiograph Can be normal in early stages when the ASD is small. Pulmonary to systemic flow ratio 2:1-normal CXR. If shunt increase( >2:1 ratio) Cardiomegaly(Right atrioventricular enlargement). Apex is rounded and elevated Aortic knuckle is inconspicuous Pulmonary bay is bulged pulmonary plethora   Features of Eisenmenger reaction

22. Complication-   Eisenmenger syndrome . Paradoxical emboli Cardiac conduction defects, e.g. atrial fibrillation, flutter. Treatment - Transcatheter occlusion by septal occludal device. Surgical-for large defect.

23. Patent ductus arteriosus (PDA) Short conical tubular connection linking the concavity of aortic arch just distal to left subclavian artery. ~1 in 2000 full-term neonates F:M of 2:1; 7.9% of all CHD. C/F- dyspnea,failure to t h rive,differential cyanosis,continuous machine-like murmur Associations VSD,ASD, Coarctation of aorta,pulmonary atresia,vascular ring

24. Plain radiograph Mild to moderate cardiac enlargement Enlargement of left atrium, left ventricle,ascending aorta and aortic arch(proximal to s h unt ) may be dilated. Pulmonary plethora if the shunt is large. Smooth outline of the aortic knuckle and upper descending aorta may be interrupted by the 'bump' of the ductus. Filling of normal angle between AoA and MPA If shunt reversal occurs- Eisenmenger reaction In cases of long-standing severe pulmonary hypertension with Eisenmenger reaction, calcification of ductus may be seen

25. 1

26. Patent ductus arteriosus (PDA) In the majority of cases there is spontaneous closure Medical   Indomethacin Endovascular   Various closure devices Surgical   Clipping or ligation to close Complications Eisenmenger phenomenon

27. Tetralogy of Fallot 12% of all CD and 4.6% of cyanotic CD. Characterised by - Large ventricular septal defect (VSD) Aortic root overriding VSD Stenosis of right ventricular outflow tract( infundibulum and pulmonary valve) Late right ventricular hypertrophy clinical Clinical Feature- Acyanotic - if PS small Fainting spells on exertion , relief with squatting Stunted growth

28. Plain radiograph P/A VIEW-Mildly enlarged or normal sized heart Apex is rounded and elevated Pulmonary bay is concave Classically show a boot shaped heart Both lung fields are oligaemic,may be normal. Right sided aortic arch is seen in 25% cases. Enlarged ascending aorta. Laterel view-right ventricular enlargement.

29. Echocardiography Echocardiography is very useful in diagnosing the condition. Will show the VSD, the overriding aorta and the right ventricular & hypertrophy very clearly. Cardiac CT/CT coronary Angiogram MDCT is useful in demonstrating the complex cardiovascular morphology of tetralogy of Fallot, especially the anatomy of the pulmonary and coronary arteries. Cardiac MRI Has the great advantage of providing both exquisite anatomical details and functional information without ionising radiation.

30. Transposition of great arteries Second most common cyanotic cardiac anomaly, Incidence is 3.8 % of all CHD Classification D loop transposition-more common Ventriculo arterial disconcordance L loop transposition

31. D-loop transposition Two great run parallel Clinical feature- cyanosis and breatlessness

32. D loop transposition Heart shadow is slightly enlarged & rounded The pedicle is relatively narrow, classically described as appearing like an egg on a string. pulmonary plethora.

33. L loop transposition (Congenitally Corrected transposition ) Atrio ventricular and ventriculo arterial Discordance Morphological LV lies anterior & to the right Association- VSD RV failure may develop.

34. L loop transposition (Corrected transposition )

35. L loop transposition (Corrected transposition ) Show a characteristic long curve to the left heart border due to the abnormal leftward origin of the aorta No separate bulge of PA and Aortic knuckle,rather characteristic bulge on upper left heart border.

36. L loop transposition (Corrected transposition ) Echocardiography - Cardiac CT/CTA CMR Treatment - Surgery   Rashkind septostomy Atrial baffle operation

37. Ebstein anomaly An anomaly of the tricuspid valve. Displacement of the tricuspid valve toward the apex of the right ventricle Larger right atrium( atrialised portion of RV). Is often associated infundibular narrowing. Severe cases presenting in infancy with right heart failure with poor flow.

38. Chest X-ray Shows massive enlarged globular cardiomegaly (box shaped heart) Markedly enlarged RA,smoothly convex right lateral heart border pulmonary oligaemia .

39. Ebstein anomaly

40. Coarctation of aorta Characteristic shelf-like narrowing of the aorta. Male predominant Aortic arch hypoplasia Aortic arch interruption C/F- Hypertension , radio-femoral delay, cardiac failure Associated with intracranial aneurysm

41. Xray Cardiomegaly (LVE) Ascending aorta may be dilated Figure of 3 sign- enlarged LSA-narrowing-post stenotic dilatation of aorta Small and flat or low aortic knuckle Rib notcing - 1. bilateral and asymmetric (post third of 3-9 ribs) 2. Unilateral – right- if coarctation between LCC & LSA Left- aberrant RSA from below coarctation

42. Coarctation of aorta

43. Partial anomalous pulmonary venous return Wen one or more individual pulmonary vein drain into right side of atrial septum in RA/ SVC/IVC Sometimes an anomalous vein may drain into IVC below diaphragm. CXR - a curved vessel in right lower zone widening as it approach to cardiophrenic angle – scimitar syndrome

44. Total anomalous pulmonary venous return(TAPVR) An abnormal drainage anatomy of the entire pulmonary venous system. All systemic and pulmonary venous blood enters the right atrium and nothing drains into the left atrium. Pathology - embryological failure of pulmonary venous development that results in persistent patency of primitive systemic veins Type I: Supracardiac (most common) Type II: Cardiac Type III: Infracardiac Type IV: Mixed pattern

45. Supracardiac type Cardiomegaly Pulmonary plethora Wide mediastinum due to left sided ascending vein Snowman appearance or figure of 8 heart or cottage loaf heart.

46. Cardiac type

47. Infra-cardiac type Little or no cardiac enlargement Interstitial edema due to pulmonary venous obstruction The findings of normal sized heart with severe eart failure usually indicate infracardiac TAPVC

48. Assessment of the chest film in the case of known or suspected congenital heart disease: Note abdominal and cardiac situs at the start. Note the overall cardiac size. Look at the mediastinum. Look at the pulmonary vascularity. Is there any characteristic unusual shape to the heart contour that suggests a particular diagnosis’? Is there any evidence of previous surgery (e.g. thoracotomy,stemal wires or implanted prosthesis)? Note skeletal or other abnormalities

49. T h ank you all